A Case of IgA kappa Light Chain Deposition Disease and Combined Adult Fanconi Syndrome with Auer rod-like Intracytoplasmic Inclusions in Plasma Cells and Proximal Renal Tubular Cells

Jimin Kahng; Jeana Kim; Suk Joon Shin; Kyungja Han

doi:10.3343/kjlm.2007.27.4.248

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Kahng, Kim, Shin, and Han: A Case of IgA kappa Light Chain Deposition Disease and Combined Adult Fanconi Syndrome with Auer rod-like Intracytoplasmic Inclusions in Plasma Cells and Proximal Renal Tubular Cells

Original Article

Korean J Leg Med 2007;27(4):248-252.

Published online: 10 February 2007

DOI: https://doi.org/10.3343/kjlm.2007.27.4.248

A Case of IgA kappa Light Chain Deposition Disease and Combined Adult Fanconi Syndrome with Auer rod-like Intracytoplasmic Inclusions in Plasma Cells and Proximal Renal Tubular Cells

Jimin Kahng, M.D.¹, Jeana Kim, M.D.², Suk Joon Shin, M.D.³, Kyungja Han, M.D.¹

¹Department of Laboratory Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea

²Department of Hospital Pathology, The Catholic University of Korea College of Medicine, Seoul, Korea

³Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea

Received 26 January 2007 Revised 19 June 2007 Accepted 20 June 2007

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

We report a case of IgA kappa light chain deposition disease and combined adult Fanconi syndrome with Auer rod-like intracytoplasmic inclusions in plasma cells and proximal renal tubular cells in a 54-yr-old female. Cytochemical stainings revealed a strong acid phosphatase activity of the inclusions and weak periodic acid-Schiff positivity, whereas the reactions for peroxidase and α-naphthyl acetate esterase were negative. An immunostaining verified IgA-kappa inside the plasma cells. Kidney biopsy revealed Bence Jones cast nephropathy with kappa light chain positivity, and Congo red staining was negative. Electron microscopy showed needle-shaped crystals located in tubular epithelial cells.

Keywords: Auer rod-like inclusion, Light chain deposition disease, Fanconi syndrome

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Fig. 1.

Bone marrow plasma cell morphology and special stain. (A) Atypical plasma cells with spindle-shaped, Auer rod-like intracytoplasmic inclusions (Wright stain, ×400/window ×1,000). (B) Strong acid phosphatase activity of the inclusions. Note the lack of staining in surrounding cytoplasm (acid phosphatase, ×1,000). (C) Negative in non-specific esterase stain (Non-specific esterase, ×1,000). (D) and (E) Immunohistochemical stain showing kappa-positivity (D) and lambda-negativity (E) (×1,000).

Fig. 2.

The kidney biopsy revealed Bence Jones cast nephropathy with kappa light chain positivity. (A) H-E stain, ×100. (B) Immunohistochemical stain showing IgA positivity (×1,000) and (C) kappa positivity (×1,000). (D) Congo red stain showing negativity (×100).

Fig. 3.

Ultrastructure of tubular epithelial cells in a case of IgA myeloma kidney. The figure shows needle-shaped crystals located in tubular epithelial cells (TEM) (A) ×2,000 & (B) ×40,000.

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